Von Hippel-Lindau综合征腹部病变的影像学表现  

Imaging Features in Abdominal Lesions of Von Hippel-Lindau Syndrome

作　　者：方旭[1] 边云[1] 邵成伟[1] 陆建平[1] 王莉[1] FANG Xu;BIAN Yun;SHAChengwei(Department of Radiology,Changhai Hospital,Second Military Medical University,Shanghai 200433,P.R.China)

机构地区：[1]第二军医大学附属长海医院影像医学科

出　　处：《临床放射学杂志》2019年第10期1877-1881,共5页Journal of Clinical Radiology

基　　金：国家自然科学基金项目(编号:81701689);上海申康医院发展中心临床科技创新基金资助项目(编号:SHDC22015035)

摘　　要：目的分析Von Hippel-Lindau(VHL)综合征在腹部病变中的CT和MRI影像学特点。方法回顾经临床病理确诊的12例VHL综合征患者资料,均行腹部CT或MRI平扫加增强扫描,分析总结其影像学特点。结果 8例肾癌,病理均为透明细胞癌,其中4例双肾多发,1例单肾多发,3例单肾单发。8例肾囊肿,其中6例双肾多发,2例单肾单发,2例有复杂性囊肿。6例胰腺肿瘤,其中5例神经内分泌肿瘤,1例浆液性囊腺瘤。11例胰腺多发囊肿,其中5例囊壁有钙化。2例双侧肾上腺嗜铬细胞,其中1例右侧肾上腺嗜铬细胞瘤已切除。3例VHL综合征家族遗传史。结论 VHL综合征腹部病变中表现多样,最常见是胰腺多发囊肿,另有肾癌或多发囊肿、胰腺神经内分泌肿瘤、胰腺浆液性囊腺瘤、肾上腺嗜铬细胞瘤。影像学早期发现对患者的早期治疗、延长生存期具有重要意义。Objective To analyze the CT and MRI features of Von Hippel-Lindau(VHL) syndrome in abdomen. Methods 12 cases of patients with VHL syndrome diagnosed by clinical pathology from December 2011 to April 2018,were collected. All patients underwent abdominal CT or MRI enhanced examination and their imaging features were analyzed and summarized. Results 8 cases of renal carcinoma and all of them were clear cell carcinoma,including 4 cases of bilateral renal multiple renal carcinomas,single renal multiple carcinoma in 1 case and single renal single carcinoma in 3 cases. Renal cyst in 8 cases,including bilateral multiple renal cysts in 6 cases and single renal single cyst in 2 cases. Renal complicated cyst in 2 cases. There were 6 cases of Pancreatic tumor,including neuroendocrine tumor in 5 cases and serous cystadenoma in 1 case. Multiple pancreatic cysts were present in 11 cases,of which cyst wall with calcification was seen in 5 cases. Bilateral adrenal pheochromocytoma was observed in 2 cases,of which 1 case of right adrenal gland had been removed. 3 cases had familial hereditary history of VHL syndrome. Conclusion VHL syndrome was diverse in the abdominal lesions. Pancreatic multiple cysts was the main feature. In addition,there were renal carcinoma,renal cysts,pancreatic neuroendocrine tumors,pancreatic serous cystadenoma,and adrenal pheochromocytomas. Early detection by imaging is of great significance for early treatment and prolonged survival.

关 键 词：Von Hippel-Lindau综合征 胰腺囊肿 体层摄影术 X线计算机 磁共振成像 

分 类 号：R73[医药卫生—肿瘤]